Middle ear neuroendocrine tumours (MENET) are rare and often misdiagnosed as more common conditions, such as cholesteatoma. This report describes the associated diagnostic workup and management, and reviews the existing literature regarding this rare entity. A 46-year-old woman with no prior otologic history presented with subjective chronic left sided hearing loss, aural fullness, vertigo and low frequency tinnitus. She was found to have an opacity of the left attic without tympanic membrane retraction or perforation. Initial CT imaging showed a soft tissue density mass of the attic with intact ossicles and no bony erosion. MRI imaging (with non-EPI DWI) showed a subtle restricted diffusion, raising the possibility of cholesteatoma. The lesion was removed surgically and histopathology with immunohistochemistry was consistent with a Middle Ear Neuroendocrine Tumour (grade I). Our review of the literature identifies 243 published clinical cases of MENET to date, with the majority of cases being treated with surgery alone, and published reports indicate local recurrence is commonly encountered. This case highlights the importance of histopathological confirmation in atypical cases of middle ear pathology and highlights the relevance of considering Neuroendocrine Tumours as a differential diagnosis for middle ear lesions. Neuroendocrine tumours of the middle ear, though rare, should be considered in cases of presumed cholesteatoma with atypical clinical or intraoperative findings, and the literature indicates such cases should be followed up due to the possibility of recurrence.